Herlyn-Werner-Wunderlich Syndrome—Timely Diagnosis is Important to Preserve Fertility

Abstract Background Herlyn-Werner-Wunderlich syndrome is an urogenital malformation with uterus didelphys and obstructed hemivagina with ipsilateral renal agenesis. Most of these patients present after the onset of menstruation. We describe two cases diagnosed too late to prevent the complications....

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Published in:Journal of pediatric & adolescent gynecology 2012-10, Vol.25 (5), p.e111-e112
Main Authors: Güdücü, Nilgün, MD, Gönenç, Gökçenur, MD, İşçi, Herman, MD, Yiğiter, Alin Başgül, MD, Dünder, İlkkan, MD
Format: Article
Language:English
Subjects:
Abdomen, Acute
Adolescent
Diagnosis, Differential
Female
Hematocolpos
Herlyn-Werner-Wunderlich syndrome
Humans
Hydrocolpos - congenital
Hydrocolpos - diagnosis
Hydrocolpos - surgery
Hysterectomy
Infertility
Kidney - abnormalities
Obstetrics and Gynecology
Pediatrics
Salpingectomy
Syndrome
Urogenital Abnormalities - diagnosis
Urogenital Abnormalities - surgery
Uterus - abnormalities
Vagina - abnormalities
Young Adult
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Summary:Abstract Background Herlyn-Werner-Wunderlich syndrome is an urogenital malformation with uterus didelphys and obstructed hemivagina with ipsilateral renal agenesis. Most of these patients present after the onset of menstruation. We describe two cases diagnosed too late to prevent the complications. Case The first patient presented with acute abdomen one year after the onset of menstruation and had salpingectomy due to pyosalpinx. The blind hemivagina was not recognized and she had severe endometriosis. She underwent hysterectomy 8 years later. The second patient presented with foul smelling vaginal discharge when she was 21 years old. She had a simple vaginal septum resection. Summary and Conclusion In the presence of uterine cavities in a regularly menstruating girl with dysmenorrhea, the presence of both kidneys should be checked. When unilateral renal agenesis and uterus didelphys coexist the first thing that we should remember is to confirm or refute the presence of a blind vagina.
ISSN:1083-3188
1873-4332
DOI:10.1016/j.jpag.2012.05.013